lymphocytic interstitial pneumonia in children
Introduction
Introduction to lymphocytic interstitial pneumonia in children Lymphocytic interstitia lpneumonia (LIP) is an uncommon lesion of benign hyperplasia of mature lymphocytes in the pulmonary interstitial and alveolar spaces. Because lymphocytic interstitial pneumonia is rare in adults, it is common in children and is associated with AIDS. basic knowledge The proportion of children: the incidence rate of children is about 0.008%-0.009% Susceptible people: children Mode of infection: non-infectious Complications: pulmonary fibrosis
Cause
Pediatric lymphocytic interstitial pneumonia
(1) Causes of the disease
The cause of lymphocytic interstitial pneumonia is unknown, premature birth and low immune function are often the cause, and the causes are as follows:
1. HIV infection with HIV infection leads to lymphocyte proliferation.
2. HIV has a synergistic effect with EBV infection.
3. Immunity, genetic factors associated with HLA-DRs, HLA-DRs phenotype, the relative risk of lymphocytic interstitial pneumonia is as high as 70.1%, can also be associated with many immune-mediated diseases, such as autoimmune hemolytic Anemia, chronic active hepatitis, juvenile rheumatoid arthritis, thyroiditis, systemic lupus erythematosus, myasthenia gravis and Sjögrens syndrome.
(two) pathogenesis
There are various hypotheses. First, HIV itself causes lymphatic hyperplasia. In addition, HIV is associated with EBV infection. EBV and HIV have synergistic effects. LIP is different from interstitial pneumonia and desquamative interstitial pneumonia. It is not interstitial. Pneumonia, which is a proliferative disease of the lung lymphocytes, occurs not only in HIV infection, but also in primary immunodeficiency and autoimmune diseases, including autoimmune diseases including Sjögren's syndrome, chronic active hepatitis, myasthenia gravis and Malignant anemia; immunodeficiency diseases include bone marrow transplant patients, there are reports of HIV-infected children follow-up, LIP can reach 30% to 50%, some people think that LIP can be familial, due to antigen-activated T lymphocytes, such as CD4 to produce cytokines IL-2, which causes the proliferation of tissue-damaged effector cells (CD8), has been reported in 8 cases of LIP in lung biopsy specimens, 8 cases of EBV have been found; also reported that CD8 lymphocytosis includes parotid swelling, Sjogren's syndrome and LIP, and, black patients are associated with HLA-DR5, and the relative risk of HLA-DR5 phenotype is 70.1%.
Prevention
Pediatric lymphocytic interstitial pneumonia prevention
There are no effective preventive measures for this disease, and should be detected early and treated promptly.
Complication
Pediatric lymphocytic interstitial pneumonia Complications pulmonary fibrosis
It can develop into pulmonary fibrosis with respiratory insufficiency, eventually with respiratory and circulatory failure.
Symptom
Symptoms of lymphocytic interstitial pneumonia in children Common symptoms Wet splenomegaly sputum sputum fingernails (toe) Lymph node swelling, salivary gland enlargement, difficulty breathing
The onset age is about 1 year old (5-81 months). The average survival time of HIV-infected children with LIP as the first symptom is 72 months. The onset is slow, non-specific symptoms, common cough, difficulty breathing and weight loss. Fever, weakness and chest pain, hemoptysis is less common, physical examination see systemic lymphadenopathy, salivary gland enlargement, fast heart rate, rapid breathing, cyanosis, clubbing, normal auscultation or wheezing or inhalation X-ray film showed increased lung texture or dot-like shadow, feathery infiltrating shadow at the bottom of the lung, visible nodules, small hilar lymph nodes, late stage development of pulmonary interstitial fibrosis with honeycomb lung, CT examination can show small Nodular shadows and ground-glass shadows, lung function showed reduced lung capacity, restricted ventilatory dysfunction, decreased lung compliance and diffuse dysfunction, the latter being a more sensitive indicator of disease progression.
Examine
Examination of lymphocytic interstitial pneumonia in children
1. Lymphocytosis and eosinophilia are seen in the surrounding blood.
2. Bone marrow can be seen in lymphocytes, plasma cells and eosinophils.
3. Blood biochemical examination of immunoglobulin increased, mainly IgM; a small number may be associated with hypogammaglobulinemia.
4. Blood gas analysis showed hypoxemia.
5 pathogen examination of alveolar bronchial lavage fluid can not find any pathogens, lung biopsy can be clearly diagnosed, in recent years, fiberoptic bronchoscopy and bronchoalveolar lavage can also be diagnosed.
6. Chest X-ray double lung texture increased or dot-like shadow, the lung base is obviously feathery, and the late stage is pulmonary interstitial fibrosis, which is a honeycomb lung shadow.
7. Pulmonary function shows restrictive ventilatory disorder, decreased lung compliance and decreased diffuse function.
Diagnosis
Diagnosis and diagnosis of lymphocytic interstitial pneumonia in children
diagnosis
1. The age of onset of the disease is around 1 year old, and there is a history of HIV infection.
2. Clinical manifestations Symptoms are insidious, slow, common symptoms are cough, difficulty breathing and weight loss, and a few have fever, weakness and chest pain.
3. Signs can be seen in the superficial lymph nodes, salivary glands enlarge, cyanosis, fast heart rate, rapid breathing, lung auscultation can have wheezing or inhalation wet snoring, some children can have clubbing, toe And liver, splenomegaly, the above characteristics combined with laboratory-assisted examination can confirm the diagnosis.
Differential diagnosis
According to the history of HIV infection, chronic progressive disease, X-ray and laboratory examination of lung biopsy pathology and other pathogen-induced interstitial pneumonia can make a diagnosis, the author has seen a 12-year-old boy LIP (pathological confirmation), X-ray It appears as a bilateral small honeycomb shadow, HIV-negative, and LIP needs to be differentiated from severe lymphatic invasive disease.
1. Identification with obvious lymphatic invasive diseases such as exogenous allergic alveolitis, angioimmunoblastic lymphoma, diffuse lymphoproliferative and follicular bronchiolitis.
2. In patients with immune injury (such as AIDS), Pneumocystis carinii, mycobacterial infection and CMV infection should be identified.
3. Lymphoma associated with bronchial lymphoid tissue Identification of primary lung lymphoma 95% is a low-grade malignant bronchial-associated lymphoid B-cell lymphoma, mostly localized, surgically resectable, limited to the lungs with a better prognosis There is a poor prognosis in patients with extrapulmonary manifestations.
